Molecular defects of ATP-sensitive potassium channels in congenital hyperinsulinism

Show-Ling Shyng, Jeremy D. Bushman, Emily B. Pratt, Qing Zhou

Research output: Chapter in Book/Report/Conference proceedingChapter

2 Citations (Scopus)

Abstract

The beta- cell ATP- sensitive potassium (KATP) channel plays a key role in regulating insulin secretion by linking glucose metabolism to cell excitability. The channel is a protein complex composed of four Kir6.2 inwardly rectifying potassium channel subunits and four sulfonylurea receptor 1 subunits encoded by KCNJ11 and ABCC8, respectively. KATP channel function in beta- cells is dependent on correct expression as well as proper gating of the channel by metabolic signals at the plasma membrane. Mutations in ABCC8 or KCNJ11 that perturb channel expression or gating can reduce channel function and lead to congenital hyperinsulinism. This chapter reviews recent advances in understanding the pathophysiology of mutant KATP channels from in vitro functional studies.

Original languageEnglish (US)
Title of host publicationMonogenic Hyperinsulinemic Hypoglycemia Disorders
PublisherS. Karger AG
Pages30-42
Number of pages13
Volume21
ISBN (Print)9783805599443, 9783805599436
DOIs
StatePublished - Feb 16 2012

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Congenital Hyperinsulinism
KATP Channels
Defects
Sulfonylurea Receptors
Inwardly Rectifying Potassium Channel
Cell membranes
Metabolism
Potassium
Adenosine Triphosphate
Insulin
Glucose
Cell Membrane
Mutation
Proteins

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Shyng, S-L., Bushman, J. D., Pratt, E. B., & Zhou, Q. (2012). Molecular defects of ATP-sensitive potassium channels in congenital hyperinsulinism. In Monogenic Hyperinsulinemic Hypoglycemia Disorders (Vol. 21, pp. 30-42). S. Karger AG. https://doi.org/10.1159/000334485

Molecular defects of ATP-sensitive potassium channels in congenital hyperinsulinism. / Shyng, Show-Ling; Bushman, Jeremy D.; Pratt, Emily B.; Zhou, Qing.

Monogenic Hyperinsulinemic Hypoglycemia Disorders. Vol. 21 S. Karger AG, 2012. p. 30-42.

Research output: Chapter in Book/Report/Conference proceedingChapter

Shyng, S-L, Bushman, JD, Pratt, EB & Zhou, Q 2012, Molecular defects of ATP-sensitive potassium channels in congenital hyperinsulinism. in Monogenic Hyperinsulinemic Hypoglycemia Disorders. vol. 21, S. Karger AG, pp. 30-42. https://doi.org/10.1159/000334485
Shyng S-L, Bushman JD, Pratt EB, Zhou Q. Molecular defects of ATP-sensitive potassium channels in congenital hyperinsulinism. In Monogenic Hyperinsulinemic Hypoglycemia Disorders. Vol. 21. S. Karger AG. 2012. p. 30-42 https://doi.org/10.1159/000334485
Shyng, Show-Ling ; Bushman, Jeremy D. ; Pratt, Emily B. ; Zhou, Qing. / Molecular defects of ATP-sensitive potassium channels in congenital hyperinsulinism. Monogenic Hyperinsulinemic Hypoglycemia Disorders. Vol. 21 S. Karger AG, 2012. pp. 30-42
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